64 YO lady THBSO for prolapse At gross : A 3 cm endometrial polyp in the fundus

Transcription

1 Case 6

2 64 YO lady THBSO for prolapse At gross : A 3 cm endometrial polyp in the fundus

3 Numerous irregular, large glands with leaf-like pattern Large glands with broad-based papillary infolding into the lumen Implantation base of the polyp : myometrium

4 Stromal cellularity is accentuated around the glands forming hypercellular cuffs with less cellular mesenchyme away from the glands

5 stromal cells: hypercellularity, one or two mitoses, plump nuclei epithelium : columnar, no atypical endometrial epithelium

6 Diagnosis?

7 Mullerian adenosarcoma

8 WHO 2003 mixed mullerian tumors Benign epithelium Malignant epithelium Benign stroma Malignant stroma Adenofibroma Adenomyoma (APA) Adenosarcoma Carcinofibroma (?) Carcinosarcoma

9 Adenosarcoma 8% of all uterine sarcomas At any age but most are postmenopausal (14-89 years, median 60) Vaginal bleeding «Recurrent endometrial polyp» (5%) : retrospectively interpreted as adenosarcoma After pelvic radiation or Tamoxifen therapy Clement and Scully, Hum Pathol 1990

10 Gross Polypoid or papillary mass 1-17 cm (5 cm) Grossly can not be distinguished from endometrial polyp Located : endometrium 83% cervix 7% myometrium 4% multiple site 7%

11 Architecture : phyllodes, leaf-like, intraglandular polypoid projection

12 Architecture : phyllodes

13 Epithelial component : Dilated, cystic glands

14 Epithelium : proliferative endometrioid without atypia with frequent metaplasia (mucinous, squamous, ciliated

15 Stroma : ESS or fibrosarcoma atypia and mitoses (2-40/10HPF)

16 Hypercelluarity is more intense around the glands with periglandular cuffing

17 Sarcomatous component Heterologous elements 24% of cases Rhabdomyosarcomatous differentiation, bone, cartilage or fat Sex-cord like differentiation 13% of cases

18 Myometrial invasion : 15-25% of cases

19 Sarcomatous overgrowth Defined as the presence of pure sarcoma, usually high grade, without a glandular component, occupying at least 25% of the tumor surface 8% of cases After many recurrences or be seen de novo Myometrial invasion 60% (sarcoma)

20

21

22 Adenosarcoma IHC Mesenchymal component ( = ESS) Soslow and Oliva 2008 Aggarwal et al 2012 ER, PR 80% (57% if sarcomatous overgrowth) CD10 82% (28% if sarcomatous overgrowth) Ki % (cuffing) (30% if sarcomatous overgrowth) WT1 80% Desmin 32% AE1/AE3 25% CD34 35% but patchy

23 Adenosarcoma differential diagnosis Mullerian adenofibroma : phyllodes and leaf like pattern no mitoses (< 2 mitoses/10hfp) no atypia, no hyercellularity of stroma or cuffing no heterologous element Differential is impossible on curettage : adenofibroma = well differentiated adenosarcoma for Gallardo and Prat, /55 cases : no atypia with < 2mitoses/10HPF on curettage and typical adenosarcoma on HT

24 Adenosarcoma differential diagnosis Endometrial polyp no phyllodes or leaf-like pattern no periglandular cuffing recurrence of large endometrial polyp

25 Adenosarcoma prognostic Tannet et al, 2013 Myoinvasion : 15-25% of cases 40-60% if sarcomatous overgrowth Local recurrence : 25% of cases usually > 5 years following hysterectomy 76% of cases if sarcomatous overgrowth

26 Adenosarcoma prognostic Distance metastasis : 5% of cases Recurrences 13% no myoinvasion 46% if myoinvasion 70% if sarcomatous overgrowth Gallardo and Prat, 2009 Most important prognostic factors : myometrial invasion sarcomatous overgrowth

27 Adenosarcoma prognosis Arend et al, 2010 Tanner et al, % stage I at diagnosis (20% with extrauterine disease) Dead of disease : 25% of patients 71% if sarcomatous overgrowth Five-year survival : 71% for stage I 48% for stage III

28 Adenosarcoma treatment Tanner et al 2013, Sutton, 2013 Surgical staging with hysterectomy Oophorectomy should be considered (low frequency of ovarian extension but for hormonal suppression: no influence on OS) No staging lymphadenectomy No adjuvant therapy for stage I, resected tumors Adjuvant therapy for high stage and sarcomatous overgrowth (sarcoma regimens)

29 TAKE HOME MESSAGES Mullerian adenosarcoma Recurrent endometrial polyp : be careful Phyllodes and leaf-like pattern Periglandular cuffing Atypia and mitoses Look for myoinvasion and sarcomatous overgrowth